By Ahmad AlMasri Definition defined as mean pulmonary artery pressure of 25 mmHg at rest as measured at right heart catheterisation N ormal mean pulmonary artery pressure ID: 917710
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Slide1
Pulmonary Hypertension(PH)
By: Ahmad Al-Masri
Slide2Definition defined as
mean
pulmonary artery
pressure of
≥
25 mmHg
at rest
,
as measured at right heart catheterisation.
N
ormal
mean
pulmonary artery
pressure?!
15
mm Hg
Slide3Classifications
Pulmonary
arterial
hypertension
Pulmonary venous hypertension
PH
caused
by
respiratory
disorders
& /or hypoxaemia
PH
caused by
chronic thromboembolic disease
Miscellaneous
Slide4Pulmonary arterial hypertension
Primary
(
Idiopathic
) :
sporadic & familial… (BMPR2 gene)!!
Typically young female (20-30) Secondary: connective tissue disease (systemic sclerosis) congenital systemic to pulmonary (left to right) shunts (VSD,ASD,PDA)portal hypertensionHIV infection various drugs or toxinspersistent pulmonary hypertension of the newborn
found in:
75
% of the familial cases
25
% of sporadic cases
Slide5Cont.
Pulmonary
arterial
hypertension will cause damage to Endothelial cells in pulmonary a.
release
Endotheline-1
SerotoninThromboxan (Less production)
Nitric Oxide
Prostacyclin
Pulmonary artery
constriction
Smooth muscle
Hypertrophy
Pulmonary artery
dilatation
I
nhibit
smooth muscle
Hypertrophy
Worsening the Pulmonary hypertension
Vicious cycle
Slide6Pulmonary venous hypertension
Left-sided atrial or ventricular heart
disease
(
eg
. hypertrophy, atrial
myxoma
,etc..) Left-sided valvular heart disease (eg. Mirtal stenosis)Pulmonary veno-occlusive diseasePulmonary capillary haemangiomatosis (very rare)
Slide7Cont.
Left Heart Disease
(Pulmonary BVs are normal & undamaged)
Left-side of heart unable to pump blood efficiently
Backup of blood in pulmonary veins and capillary beds
↑ Pressure in pulmonary artery
Slide8PH caused by respiratory disorders & /or hypoxaemia
COPD
Diffuse
parenchymal
lung disease
Sleep-disordered
breathing
Alveolar hypoventilation disordersChronic exposure to high altitudeNeonatal lung diseaseAlveolar capillary dysplasiaSevere kyphoscoliosis
Slide9Cont.
respiratory disorders & /or hypoxaemia
Hypoxic
vasoconstriction
of pulmonary arterioles
↑ Pulmonary vascular resistance
(harder for Rt. Ventricle to pump blood)
Slide10PH caused by chronic thromboembolic disease
Thromboembolic obstruction
of the
proximal pulmonary
arteries
In
situ thrombosis
Sickle cell disease
Emboli
BLOCK
pulmonary vessels
↑ resistance to blood flow
Endothelial cells will release
Histamine
Serotonin
Constrict pulmonary arterioles
↑ pulmonary BP
Slide11MiscellaneousInflammatory conditions
Extrinsic
compression of central pulmonary
veins…
eg
. Tumour
Slide12Pathological featureshypertrophy of
the media of
the vessel
wall
Intimal fibrosis
plexiform
lesions?!
(group of capillaries placed together, that arise as complication of long standing pulmonary hypertension)
Slide13Consequences
Lung
pulmonary edema
Heart Rt. Ventricle hypertrophy
O2 Demand exceeds O2 supply
Rt. Sided heart failure
(
Cor Pulmonule)
Blood backup in venous system:
↑JVD
Hepatomegaly
Ascites
Legs edema
Left ventricle receive less blood
To compensate it will pump harder & faster
Slide14Clinical features
Symptoms:
- Breathlessness - chest pain
- Fatigue
- palpitation
- Syncope
(with severe disease)Signs:accentuation of pulmonary component of the second heart sound (S2) m.c.Right ventrical heave (right ventricular hypertrophy) elevation of the jugular venous pulse (prominent ‘a’ wave if in sinus rhythm)right ventricular third heart sound.Tricuspid regurgitation
Slide15Investigations
CXR?!
Enlarged pulmonary arteries
ECG?!
right
axis
deviation
due to Rt. Ventricle hypertrophyEchocardiogram?!1) Dilated pulmonary artery2) Dilatation/hypertrophy of RA and RV3) Abnormal movement of IV septum (due to increased right ventricular volume)Right heart catheterization?!increased pulmonary artery pressureV/Q scan… when?!If the cause is not revealed (neither heart nor lung), to know is it due PE or is it primary pulmonary hypertension
Slide16Cont.
PFT?!
most
pulmonary function
parameters are
normal in
PH
(if it’s not caused by respiratory disorders) , but the DLCO (Diffusing capacity of the lungs for carbon monoxide) decreases. Know that a very low DLCO is associated with a poor prognosis in patients with PH.
What are the 1st tests you order
when you suspect PH?!
What
follow-up test is done if the
1
st
tests are
suggestive
?!
ECG
& an
echocardiogram
.
Right heart catheterization to confirm the diagnose
Slide17Management
1)
Exercise
: improve the functional class of patients greatly but does not change
the
hemodynamics
.
2) Unless there is an increased risk of bleeding anticoagulants mainly warfarin. Our goal is to reach INR of 2.0.3) Diuretics are recommended for fluid retention.4) Oxygen for those with chronic hypoxaemia.5) Vasodilators6) Lung transplantation
Slide18Vasodilator agents
1) Oral
Calcium
Channel
Blockers
.. vasodilators
of choice
(eg. ifedipine, amlodipine & diltiazem)2) Oral Endothelin Receptor Antagonists (eg. Bosentan)3)IV Prostacyclin (eg. Epoprostenol)4) Inhaled PDE (phosphodiesterase) Inhibitors type 5
Slide19Questions ??